Extraspinal sacrococcygeal anaplastic ependymoma: A case report.

Introduction and importance: Ependymomas are glial tumors typically located in the central nervous system. Extraspinal ependymomas, which occur outside the CNS, are exceedingly rare and present significant diagnostic challenges. These tumors most commonly arise in the sacrococcygeal region, often being misdiagnosed as benign or malignant soft tissue masses. Case presentation: A case of an extraspinal sacrococcygeal subcutaneous ependymoma in a young female adult is presented, highlighting the importance of imaging, histological examination, and complete surgical resection in the management of such tumors. Clinical discussion: Extraspinal ependymomas are classified into three histological grades, with Grade 3 (anaplastic) ependymomas being more aggressive and associated with a higher risk of metastasis. Although complete surgical excision is the primary treatment, adjuvant radiotherapy may be required for high-grade tumors or incomplete resections. Conclusions: Extraspinal ependymoma is a rare, potentially malignant tumor that may mimick benign subcutaneous masses, leading to delayed diagnosis. Preoperative MRI is essential for surgical planning and achieving complete resection, which is key for curative treatment. Long-term follow-up is recommended given to the risk of late recurrence and metastases, especially in high-grade forms.